Spectrum of pericardial disease: part I

Pericardial disease is a common disorder seen in varying clinical settings and may be the first manifestation of an underlying systemic disease. It may be due to multiple causes. Epidemiologic studies are lacking, and the exact incidence and prevalence are unknown. New diagnostic techniques have improved diagnosis, allowing early diagnosis and management. There are few randomized data to guide physicians in the management of pericardial diseases. Part I of our review focuses on the current state of knowledge and management of the more common pericardial diseases: acute pericarditis, pericardial effusion, cardiac tamponade, chronic pericarditis and relapsing pericarditis.     

A review of pericardial diseases: clinical, ECG and hemodynamic features and management

Pericardial diseases are common, have multiple causes, and are often misdiagnosed. Physicians need to recognize the characteristic and distinguishing features of the three most important pericardial conditions: acute pericarditis, cardiac tamponade, and constrictive pericarditis. In these conditions, proper diagnosis and appropriate management can significantly reduce morbidity and mortality.     

Spectrum of pericardial disease: part II

Pericardial disease is a common disorder seen in varying clinical settings, and may be the first manifestation of an underlying systemic disease. In part I, we focused on the current knowledge and management of the more common pericardial diseases: acute pericarditis, pericardial effusion, cardiac tamponade, chronic pericarditis and relapsing pericarditis. In part II, we will focus on the knowledge and management of pericardial involvement in chylous pericardial effusion cholesterol pericarditis, radiation pericarditis, pericardial involvement in systemic inflammatory diseases, autoreactive pericarditis, pericarditis in renal failure, pericardial constriction and effusive constrictive pericarditis.     

Cardiac tamponade in systemic lupus erythematosus: an unusual initial manifestation

I have described a patient in whom cardiac tamponade occurred as the initial clinical manifestation of SLE. Although pericarditis is a common clinical entity in SLE, cardiac tamponade with this disease is rare. If suspected, the diagnosis can be made by the proper selection of tests of serum and pericardial fluid, which should include the search for pericardial LE cells. This report emphasizes the importance of screening for connective tissue disease in patients with pericarditis.     

Purulent pericarditis misdiagnosed as septic shock

BACKGROUND: Septic shock is common, with approximately 200,000 cases recognized annually. This syndrome is so well characterized that when a patient is febrile and in shock, septic shock may be diagnosed without regard to alternative possibilities. Purulent pericarditis is a relatively rare disorder in which fever and hypotension are common. Classic signs and symptoms, such as chest pain, pericardial friction rub, pulsus paradoxus, and elevation of jugular venous pressure, are seen in only 50%. METHODS: In this report, we describe four patients in whom purulent pericarditis and pericardial tamponade was initially misdiagnosed as septic shock. During a 3-month period, three men and one woman (mean age, 44.5 years) came to Kern Medical Center with purulent pericarditis and pericardial tamponade. These cases represented 13% of patients admitted with a diagnosis of septic shock. RESULTS: All patients were bacteremic, and the classic findings of pericardial tamponade were absent or relatively subtle. Hemodynamic findings of elevated systemic vascular resistance, low cardiac output, and normal pulmonary artery occlusion pressure were critical to the diagnosis. CONCLUSIONS: Consideration of purulent pericarditis is important in cases diagnosed as septic shock. Clinicians should be aware that patients with purulent pericarditis may not exhibit classic signs and symptoms, and a high index of suspicion is necessary for appropriate management.     

Echocardiographic diagnosis of effusive-constrictive pericarditis due to staphylococcal pericarditis after cardiac surgery

Two weeks after coronary artery bypass surgery, a 43-year-old man was readmitted with fever, pneumonia, left pleural effusion, and pericarditis. Echocardiography showed a localized posterior pericardial effusion, pericardial thickening, and bulging of the ventricular septum toward the left ventricle. Right-sided catheterization indicated pericardial constriction. Effusive-constrictive pericarditis was confirmed at surgery. Cardiac imaging played an important role in diagnosis of this unusual complication of cardiac surgery.     

Pericardial Disease: Diagnosis and Management

Pericardial diseases can present clinically as acute pericarditis, pericardial effusion, cardiac tamponade, and constrictive pericarditis. Patients can subsequently develop chronic or recurrent pericarditis. Structural abnormalities including congenitally absent pericardium and pericardial cysts are usually asymptomatic and are uncommon. Clinicians are often faced with several diagnostic and management questions relating to the various pericardial syndromes: What are the diagnostic criteria for the vast array of pericardial diseases? Which diagnostic tools should be used? Who requires hospitalization and who can be treated as an outpatient? Which medical management strategies have the best evidence base? When should corticosteroids be used? When should surgical pericardiectomy be considered? To identify relevant literature, we searched PubMed and MEDLINE using the keywords diagnosis, treatment, management, acute pericarditis, relapsing or recurrent pericarditis, pericardial effusion, cardiac tamponade, constrictive pericarditis, and restrictive cardiomyopathy. Studies were selected on the basis of clinical relevance and the impact on clinical practice. This review represents the currently available evidence and the experiences from the pericardial clinic at our institution to help guide the clinician in answering difficult diagnostic and management questions on pericardial diseases.CMR = cardiac magnetic resonance imaging; CT = computed tomography; CYP = cytochrome P450; ECG = electrocardiographic; ESC = European Society of Cardiology; IVC = inferior vena cava; LV = left ventricular; NSAID = nonsteroidal anti-inflammatory drug; RA = right atrium; RV = right ventricleThe pericardium is a thin covering that separates the heart from the remaining mediastinal structures and provides structural support while also having a substantial hemodynamic impact on the heart. The pericardium is not essential—normal cardiac function can be maintained in its absence—however, diseased pericardium presenting clinically as acute or chronic recurrent pericarditis, pericardial effusion, cardiac tamponade, and pericardial constriction can be challenging to manage and life-threatening in some cases. The etiology of pericardial disease is often difficult to determine or remains idiopathic. However, microorganisms, including viruses and bacteria; systemic illnesses, including neoplasia, autoimmune disease, and connective tissue disease; renal failure; previous cardiac surgery; previous myocardial infarction; trauma; aortic dissection; radiation; and, rarely, drugs have been associated with pericardial diseases.The diagnosis and management of pericardial diseases remain challenging because of the vast spectrum of manifestations and the lack of clinical data on which to base guidelines by the American College of Cardiology and the American Heart Association. However, the European Society of Cardiology (ESC) published guidelines on pericardial disease in 2004.¹ This review aims to describe the methods of diagnosing and managing major pericardial syndromes on the basis of the literature and the clinical experience of our pericardial clinic. Searches were performed on PubMed and MEDLINE using the keywords diagnosis, treatment, management, acute pericarditis, relapsing or recurrent pericarditis, pericardial effusion, cardiac tamponade, constrictive pericarditis, and restrictive cardiomyopathy. No date limitations were set. Studies were selected on the basis of clinical relevance and the impact on clinical practice.     

Cardiac tamponade as a manifestation of tuberculosis

Tuberculosis has been increasing in incidence in recent years. Pericardial involvement and pericardial effusions are well-documented and may result in pericardial tamponade. Despite this, large pericardial effusions are uncommon, and manifestation as cardiac tamponade is rare. We report two cases of tuberculous pericarditis in which the initial feature was tamponade. Since the diagnosis of tuberculosis may be delayed due to the slow-growing nature of the bacterium, physicians need to be aware of this possibility and consider the use of modern diagnostic techniques that may permit an earlier diagnosis.     

The constricted heart

Often indistinguishable from restrictive cardiomyopathy and hepatic cirrohis, clinical acumen is essential in the recognition and diagnosis of constrictive pericarditis. A thorough medical history should rule out infectious disease exposure. A physical examination may include variable signs such as Kussmaul's sign, pulsus paradoxus, and pericardial knock, while jugular venous distention is of cardinal significance in eliminating liver cirrhosis as the cause of ascites. A complete physical examination, appropriate imaging studies, and cardiac catheterizaiton are crucial for proper diagnosis and prompt treatment of constrictive pericarditis.     

Noninvasive imaging techniques of constrictive pericarditis

Constrictive pericarditis (CP) is the result of scarring and loss of elasticity of the pericardial sac, resulting in external impedance of cardiac filling. It can occur after virtually any pericardial disease process. Patients typically present with signs and symptoms of right heart failure and/or low cardiac output. An important pathophysiological hallmark of CP is exaggerated ventricular interdependence and impaired diastolic filling. Echocardiography is the initial imaging modality for diagnosis of CP. Unfortunately, no echocardiographic sign or combination of signs is pathognomonic for CP. CT scan and cardiac MRI are other imaging techniques that can provide incremental diagnostic information. CT scan can easily detect pericardial thickening and calcification, while cardiac MRI provides a comprehensive evaluation of the pericardium, myocardium and cardiac physiology. Occasionally, a multimodality approach needs to be considered for the conclusive diagnosis of CP.     

Diagnostic issues in the clinical management of pericarditis

Aims: To review the current major diagnostic issues on the diagnosis of acute and recurrent pericarditis. Methods: To review the current available evidence, we performed a through search of several evidence‐based sources of information, including Cochrane Database of Systematic Reviews, Clinical Evidence, Evidence‐based guidelines from National Guidelines Clearinghouse and a comprehensive Medline search with the MeSH terms ‘pericarditis’, ‘etiology’ and ‘diagnosis’. Results: The diagnosis of pericarditis is based on clinical criteria including symptoms, presence of specific physical findings (rubs), electrocardiographical changes and pericardial effusion. Although the aetiology may be varied, most cases are idiopathic or viral, even after an extensive diagnostic evaluation. In such cases, the course is often benign following anti‐inflammatory treatment, and management would be not affected by a more precise diagnostic evaluation. A triage of pericarditis can be safely performed on the basis of the clinical and echocardiographical presentation. Specific diagnostic tests are not warranted if no specific aetiologies are suspected on the basis of the epidemiological background, history and presentation. High‐risk features associated with specific aetiologies or complications include: fever > 38 °C, subacute onset, large pericardial effusion, cardiac tamponade, lack of response to aspirin or a NSAID. Conclusions: A targeted diagnostic evaluation is warranted in acute and recurrent pericarditis, with a specific aetiological search to rule out tuberculous, purulent or neoplastic pericarditis, as well as pericarditis related to a systemic disease, in selected patients according to the epidemiological background, presentation and clinical suspicion.     

Multimodality imaging for the diagnosis and treatment of constrictive pericarditis

ABSTRACT

Introduction: Constrictive pericarditis can result in debilitating congestive right heart failure and has been considered an important cause of morbidity and mortality in patients with cardiovascular disease. Multimodality imaging continues to play a fundamental role in the individual approach to diagnosis, management, and prognosis of patients with this clinical syndrome.

Areas covered: This article gives an overview of the clinical spectrum of constrictive pericardial diseases and the role of multimodality imaging in the diagnosis of constrictive pericarditis. There is a focus on the emerging role of cardiac magnetic resonance (CMR) for the diagnosis, management, and prognostication of patients with constrictive pericarditis based on more recent case series, retrospective and prospective studies, which have helped to define the role of CMR.

Expert opinion: Advanced multimodality imaging assists with identification of both overt and subclinical pericardial inflammation. This allows the pericardiologist to recognize patients with potentially reversible disease, trial medical therapy, and thereby avoid mechanical removal of the pericardium. Further, pericardial characterization by CMR has provided novel information about the natural history of these pericardial conditions, which can help tailor therapy and improve prognosis.     

Hypothyroidism presenting as acute cardiac tamponade with viral pericarditis

This report describes the case of a young woman who presented to an emergency department with severe abdominal pain and shock. The patient was found to have pericardial tamponade due to a massive pericardial effusion. On further evaluation, the etiology of this effusion was considered to be secondary to hypothyroidism with concominant acute viral pericarditis leading to a fulminant tamponade. The presentation, differential diagnosis, and management of pericardial effusion and tamponade secondary to hypothyroidism and viral pericarditis are discussed. The diagnosis of hypothyroidism in conjunction with acute viral pericarditis should be considered in patients presenting with unexplained pericardial effusion and tamponade.     

Haemophilus influenzae pericarditis with tamponade as the initial presentation of systemic lupus erythematosus

Although cardiac tamponade is an important and emergent complication of systemic lupus erythematosus (SLE), purulent pericarditis is rare despite the high frequency of pericardial effusion in SLE. We describe the first SLE case of Haemophilus influenzae type-f pericarditis with cardiac tamponade with SLE as the initial presentation. The pathophysiology and therapy are discussed.     

Thoracoscopic pericardiotomy for management of purulent pneumococcal pericarditis in a child

Purulent pericarditis is an extremely rare complication of pneumococcal pneumonia in children that may result in to cardiac tamponade. While image-guided pericardiocentesis is the treatment of choice for such a condition, it may fail in the presence of thick pus; loculations and thoracoscopic pericardiotomy are useful procedures for such situations. Herein, we report such a case involving a 6-year-old boy who presented with purulent pneumococcal pericarditis that was managed with thoracoscopic pericardiotomy and who recovered well. Thoracoscopic pericardiotomy is a safe procedure that allows effective drainage under vision, pericardial biopsy for diagnosis, and a simultaneous opportunity to perform thoracoscopic pleural drainage.     

Constrictive pericarditis: assessment of current diagnostic procedures

Constrictive pericarditis frequently poses a diagnostic challenge because of its varied manifestations. Accurate diagnosis is essential, however, because surgical decortication may yield excellent clinical results. Although new diagnostic procedures have helped the clinician to diagnose constrictive pericarditis, the initial clinical suspicion of this diagnosis must be high for appropriate interpretation of these tests. Echocardiography is useful, primarily for distinguishing various other cardiac abnormalities that may simulate constrictive pericarditis. Computed tomography is a valuable procedure for assessment of pericardial thickening. In addition, evaluation of early diastolic filling by computerized digitization in conjunction with echocardiography, angiography, and invasive hemodynamics shows promise as a diagnostic tool. Even with these new diagnostic aids, distinguishing constrictive pericarditis from restrictive cardiomyopathy may be difficult and, in some cases, may necessitate an exploratory operative procedure.     

CT Imaging of the Pericardium

Pericardial disease can be challenging to diagnose, and imaging can play a useful role in confirming or even suggesting the diagnosis. Computed tomography (CT) is a particularly appealing option for investigating pericardial disease in many patients because the differential diagnosis for symptoms of acute pericarditis or constrictive pericarditis often includes other diseases which are also well assessed with CT. In addition, many patients will have findings of pericardial disease manifest on CT imaging for other suspected diseases, and these findings can be missed if careful attention is not paid to the pericardium. CT also can play an important role in evaluating specific pericardial lesions, such as cysts, tumors, and abscesses. We will review findings of various pericardial diseases on CT with illustrative cases.     

MR imaging findings in 76 consecutive surgically proven cases of pericardial disease with CT and pathologic correlation

To describe findings of patients with surgically confirmed pericardial disease on state of the art MR sequences. Retrospective review was performed for patients who underwent pericardiectomy and preoperative MR over a 5 year period ending in 2009. Patients' records were reviewed to confirm the diagnosis of chronic recurrent pericarditis, constrictive pericarditis, or pericardial tumor. MR imaging findings of pericardial thickness, IVC diameter, presence or absence of pericardial or pleural effusion, pericardial edema, pericardial enhancement, and septal "bounce" were recorded. Patients with constriction had a larger IVC diameter (3.1 ± 0.4 cm) than patients with recurrent pain and no constriction (2.0 ± 0.4 cm). Mean pericardial thickness for the 16 patients with chronic recurrent pericarditis but no evidence of constriction was 4.8 ± 2.9 mm. Mean pericardial thickness for patients with constriction was 9.2 ± 7.0 cm with calcification, and 4.6 ± 2.1 cm without calcification. 94% of patients with chronic recurrent pericarditis had gadolinium enhancement of the pericardium, while 76% of patients with constriction had pericardial enhancement. Septal "bounce" was present in 19% of chronic recurrent pericarditis cases and 86% of constriction cases. 5 patients had a pericardial neoplasm, 1 of which was not identified preoperatively. State of the art MR techniques can identify significant and distinct findings in patients with chronic recurrent pericarditis, constrictive pericarditis, and pericardial tumors.     

Acute Pericarditis: Best Practices for Nurse Practitioners

Acute pericarditis (AP) is inflammation of the outermost layer of the heart due to infectious or noninfectious etiologies that result in increased pericardial vascular permeability, cardiac motion restriction, and augmented electrophysiology. It is a clinical diagnosis based on the presence of at least 2 of 4 clinical manifestations: pleuritic chest pain, pericardial friction rub, widespread ST elevation or PR depression, and new or worsening pericardial effusion. Nurse practitioners in primary and acute care settings need to recognize the hallmark finding of new global ST elevation or PR depression on electrocardiogram, appropriately prescribe nonsteroidal antiinflammatory drugs while minimizing side effects, and coordinate interdisciplinary care to reduce morbidity and mortality of AP in adult and older adult populations.     

An Unusual Cause of Severe Dyspnea: Diastolic Dysfunction Due to Calcific Constrictive Pericarditis

Background: Constrictive pericarditis is a rare cause of dyspnea. This disease shares many signs and symptoms with other causes of cardiac failure as well as gastrointestinal and renal diseases, making it difficult to diagnose. Case Report: We present a case of a 73-year-old woman who presented to our Emergency Department (ED) in respiratory failure after a recent history of worsening dyspnea. Constrictive pericarditis was strongly suspected on bedside ultrasonography. Computed tomography scan showed extensive pericardial calcifications and large pleural effusions, supporting the diagnoses. The patient was admitted for treatment and evaluation of constrictive pericarditis, but died of complications during cardiac catheterization. Conclusions: The etiology and physiology of constrictive pericarditis are reviewed and an ultrasound-centered approach to undifferentiated dyspnea in the ED is discussed.